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Approach to ild & update
1. Dr Nahid Sherbini
Internal Medicine & Pulmonary Consultant
King Abdulaziz Medical City / Riyadh
Certified from Harvard Medical School in Practice of clinical Research
APPROACH TO ILD
& UPDATE IN IPF MANAGEMENT
2. OBJECTIVES
• Classification of ILD or DPLD
• An algorithm to make the diagnosis
• Review common radiographic findings in ILD
• Update in Management of IPF
3. INTRODUCTION
• (ILDs) are a heterogeneous group of disorders
that are classified together because of similar
clinical, radiographic, physiologic, or pathologic
manifestations .
5. WHAT IS THE PULMONARY INTERSTITIUM?
• between the epithelial and
endothelial basement membrane
• Expansion of the interstitial
compartment by inflammation with
or without fibrosis
• Necrosis
• Hyperplasia
• Collapse of basement membrane
• Inflammatory cells
6. PATHOGENESIS
The pathogenesis of ILDs is unknown.
But more and more facts have shown that immune
cells and their cytokines play an important role in
the course of ILDs.
13. HISTORY
• Age and gender
• Duration of symptoms
• Past medical history
• Smoking history
• FH
• Prior medication use and irradiation
• Occupational and environmental exposures
• Symptoms
14. • LAM
• Tuberous sclerosis
• Pneumoconiosis
HISTORY: AGE AND GENDER
Age Gender
15. HISTORY
• Age and gender
• Duration of symptoms
• Past medical history
• Smoking history
• FH
• Prior medication use and irradiation
• Occupational and environmental exposures
• Symptoms
27. • Anti-JO-1 ab even in the absence of clinical myositis, as ILD
precedes the onset of myositis ~70% of patients with the anti-
synthetase syndrome.
40. PFT
• A restrictive defect :
(TLC), (FRC), (RV) ,(FVC) and (FEV1)
but usually the changes are in proportion to the
decreased lung volumes
41. ROLE OF BRONCHOALVEOLAR LAVAGE
• The lavage fluid is sent for cell counts, cultures for mycobacterial,
viral and fungal pathogens, and cytologic analysis.
• Virtually all patients presenting with hemoptysis and radiographic
ILD should undergo BAL to confirm an alveolar source of
bleeding and identify any infectious etiologies.
42.
43. PROBABILITY OF HISTOLOGIC DIAGNOSIS OF DIFFUSE DISEASES
Surgical
Biopsy
1. Granulomatous diseases
2. Malignant tumors/lymphangitic
3. DAD (any cause)
4. Certain infections
5. Alveolar proteinosis
6. Eosinophilic pneumonia
7. Vasculitis
8. Amyloidosis
9. EG/HX/PLCH
10. LAM
11. RB/RBILD/DIP
12. UIP/NSIP/LIP COP
13. Small airways disease
14. PHT and PVOD
Often
Sometimes
Rare
Transbronchial
Biopsy
Courtesy of Kevin O. Leslie, MD.
44. MICROSCOPIC PATHOLOGY
USUAL INTERSTITIAL PNEUMONIA
• Fibrosis with honeycombing
• Architectural destruction
• Peripheral and basal distribution
• Patchy (i.e. normal and abnormal lung)
• Fibroblastic foci
48. AVERAGE SURVIVAL DIAGNOSIS OF IPF IS
APPROXIMATELY 2.5–3.5 YEARS1 FROM
DIAGNOSIS
Onset of symptoms
Initial visit
Kaplan-Meier plot of the survival
probability in IPF patients (n=238)2
1. Ley B et al. Am J Respir Crit Care Med 2010 October 8 2. King TE et al. Am J Respir Crit Care Med 2001; 164: 1171-1181
49. ESTABLISH DIAGNOSIS
Multi-Disciplinary Team (MDT)
Discussion
Clinical
• Symptoms
• Smoking history
• Exposures
• Features of CTD
• Examination
Investigations
• CXR
• CT Thorax
• Blood tests
• Lung Function
Pathology
• Bronchoalveolar lavage
• Surgical lung biopsy
53. ASCEND: KEY INCLUSION CRITERIA
40–80 years of age
Definite UIP on HRCT, or possible UIP on HRCT plus definite or
probable UIP on surgical lung biopsy
Extent of fibrotic changes greater than extent of emphysema on
HRCT scan
FVC ≥50% and ≤90% predicted
DLCO ≥30% and ≤90% predicted
FEV1/FVC ratio ≥0.8
6MWT distance ≥150 m
King TE Jr, et al. N Engl J Med 2014;370:2083-2092.
60. ALL-CAUSE MORTALITY OVER 52 WEEKS: POOLED
DATA FROM INPULSIS®
Placebo
Nintedanib 150 mg bid
HR 0.70
(95% CI; 0.43, 1.12)
p=0.1399
Richeldi L, et al. N Engl J Med 2014;370:2071–2082.
